A system...

I think everyone has heard about the ten year old girl, Sarah Murnaghan, in Pennsylvania, who needs a lung transplant. Yesterday a judge ordered the Organ Procurement and Transplantation Network System to suspend their under-12 rule for Sarah.

This case has brought a lot of attention to organ donation and how organs are distributed. And over the past few days I've seen numerous comments from people who are not very well educated about the subject of organ donation.

This needs to change.

I need a double-lung transplant and I am learning much more than most anyone needs to know. I don't know what the ramifications of this case will be concerning the future of organ transplantation. But I do know that the system was overhauled in 2005 to increase the effectiveness of allocating organs and I'm told it has been successful at procuring more organs and reducing the amount of people dying waiting for organs. Perhaps this will be a good time for the organization to re-evaluate things and make sure the system is still running smoothly.

Perhaps this is also a good time to talk about an underlying issue here. And that is, despite children under twelve dying every day who could potentially be donors, their families are not agreeing to organ donation. Only 20 deceased lung donors in 2012 were under the age of 10.

Statistically no child should die waiting for an organ.

Live it up...

This weekend I had a visit from a childhood friend. It helped. I woke up more confident and focused this morning. She told me I have no choice but to "live it up" from here on out. I promised her I will do so... to the best of my ability.

I know what the statistics say. I know what the odds are. But, I must focus on being on the side with the best outcome and hope the outcome gets better the longer I can hold out. There are always new advancements in medicine and technology and better medications on the horizon. The intravenous epoprostenol I am currently trying wasn't even approved by the FDA until 1995. Since then it has given many patients an opportunity to live longer and for some, has provided the boost needed between diagnosis and a potential lung transplant. And, even though lung transplantation is an extremely complicated surgery with high mortality rates, there are better post-transplant drugs available now.

The weirdest thing is knowing I have a time limit. Sure, anyone can die at any time. Nothing is guaranteed.  But, to know I probably won't make it to see certain milestones is difficult to accept. This morning I read Living with death in the Guardian News, an article about four people sharing their thoughts on living with terminal illness. Holly Webber, who has cancer and was 25 at the time of the article said, "Sometimes I feel like I'm on another planet looking in on this one. I can't relate to people stressing about work.... People are so wound up, but it's such a waste of time and energy. Chill out! I hope that by reading this, someone out there will take a second to think, "I'm glad that's not me. Maybe I should worry less about the things that don't really matter.""

Like Holly, I am finding it hard to relate to the people around me. When I cruise through Facebook I find so many of my friends and family complaining about stuff that is so trivial. Or, things that seem like a crisis are really insignificant in the context of things. For the most part the wrinkles will fall out with a little time, and their life will go on just the same. People complain about their kids or their spouse... but what happens when they no longer have them in their life? People complain about things needing repaired, but what happens when they no longer have it within their capacity to repair things? People complain about traffic, but what happens when they are no longer able to drive or even manage public transportation on their own? People obsess about looks, clothes, and hair; but what happens when their body is ravaged by medication side effects that makes their hair fall out, their face flushed, causes swelling, and/or rashes on their body? People complain when they have a headache or get a common cold, but those too will pass. And of all things, people complain about getting older. What? Getting older is a privilege denied to some. To grow old... they should be thankful.

Titrating up...

Wednesday was my follow-up at the Cleveland Clinic. It was a long day but fairly simple - a walking test, in which they measure oxygen levels for six minutes of walking; blood-work; and a visit with the doctor. The good news is that I appear no worse. So, we will continue the Flolan therapy as planned and hope for the best.

Thursday, as instructed by my doctor, I raised the Flolan at home. I took Tylenol beforehand, anticipating some pain. I got the usual flushing, headache, and some pain in my calves. But, overall, it was not as bad as when I was in the hospital. That may be because they were upping it about one time each day, where here at home they may up it once a week. The jaw pain while eating (at the beginning of meals) also increased.

Being tethered to an IV pump is awkward. I'm still dependent on the oxygen, too. So, wherever I go I feel like I take up a lot of space, encumbered with tubes and other breathing paraphernalia. I must look a bit like Doctor Octopus and sound like Darth Vader. So, needless to say, between that and my lack of energy, I don't get out much.

My husband and I mix my medication each evening. We timed it the other day and found it takes a little over 32 minutes to mix and change the cartridge on the pump. And, that was on a good day. Once a week I have to change the dressing and one of the connectors that go on the Hickman. And, three times a week I have to change the extension tubing. To shower I have to cover the Hickman site and hang the pump from the towel rod.

Again I cannot express how thankful I am for all the help from family and friends. I hope everyone remembers that this is going to be a long process so please do not hesitate to keep reminding us that you can help.

And for now, I will leave you with a link to an article about Carson Smith, a wonderful lady who is also living with PVOD. The article is six years old and I am happy to report that Carson is still doing well and has been stable on medications.